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Classification

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Three types of von Willebrand's disease are recognised on the basis of ristocetin activity and other tests:

  • type I, the the classic type described by von Willebrand in 1926, where the von Willebrand factor is functionally normal but present at low levels
  • type II, a broad spectrum of diseases in which bleeding time is prolonged in almost all cases. vWF levels may increase under some conditions - like stress or pregnancy, correcting the bleeding time
  • type IIa - both plasma and platelet samples lack the intermediate and high molecular weight vWF multimers; platelets do not aggregate with ristocetin
  • type IIb - plasma lacks the highest molecular weight vWF multimers; platelets aggregate in response to low dose ristocetin
  • type III is inherited as an autosomal recessive trait. Homozygous individuals have severe bleeding problems related to marked reduction of all von Willebrand's factor parameters and of Factor VIII

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